Document Detail

Metabolic characterization of spinocerebellar ataxia type 6.
MedLine Citation:
PMID:  11176970     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disorder characterized by slowly progressive ataxia and dysarthria. The mutational basis is an expanded CAG repeat sequence within the coding regions of the CACNL1A4 gene. Basic clinical, neuroimaging, and pathological, and epidemiological features have been described in the literature. However, the metabolic features of SCA6 have not been elucidated. OBJECTIVE: To investigate the metabolic features of SCA6. PATIENTS AND METHODS: Seven patients with SCA6 and 7 healthy individuals underwent positron emission tomography using fluorodeoxyglucose F 18. RESULTS: Cerebral glucose utilization in the 7 patients with SCA6 was characterized by significant hypometabolism in widespread structures, including cortical regions and basal ganglia, as well as the cerebellar hemispheres and brainstem. CONCLUSIONS: The results of the multiple-regional brain hypometabolism suggest that brain dysfunction associated with SCA6 may not be limited to the cerebellum and inferior olive, as previously suggested by the results of other pathologic studies.
B Soong ; R Liu ; L Wu ; Y Lu ; H Lee
Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of neurology     Volume:  58     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2001 Feb 
Date Detail:
Created Date:  2001-02-22     Completed Date:  2001-03-29     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  300-4     Citation Subset:  AIM; IM    
Neurological Institute, Veterans General Hospital-Taipei, Taipei, Taiwan 112, Republic of China.
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MeSH Terms
Cerebellum / metabolism*,  radionuclide imaging
Fluorodeoxyglucose F18 / diagnostic use
Glucose / metabolism*
Middle Aged
Radiopharmaceuticals / diagnostic use
Spinocerebellar Ataxias / diagnosis,  metabolism*
Statistics, Nonparametric
Telencephalon / metabolism*,  radionuclide imaging
Tomography, Emission-Computed
Reg. No./Substance:
0/Radiopharmaceuticals; 50-99-7/Glucose; 63503-12-8/Fluorodeoxyglucose F18

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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